Giant cell arteritis
| Giant cell arteritis | |
|---|---|
| Classification and external resources | |
|
File:Gray508.png The arteries of the face and scalp. | |
| ICD-10 | M31.5-M31.6 |
| ICD-9 | 446.5 |
| OMIM | 187360 |
| DiseasesDB | 12938 |
| eMedicine | neuro/592 |
| MeSH | D013700 |
Giant cell arteritis (GCA or temporal arteritis) is an inflammatory disease of blood vessels (most commonly large and medium arteries of the head). It is a form of vasculitis.
The name (giant cell arteritis) reflects the type of inflammatory cell that is involved[1] (as seen on biopsy).
The terms "giant cell arteritis" and "temporal arteritis" are sometimes used interchangeably, because of the frequent involvement of the temporal artery. However, it can involve other large vessels (such as the aorta in "giant cell aortitis".[2] Giant cell arteritis of the temporal artery is referred to as "temporal arteritis," and is also known as "Cranial arteritis" and "Horton's disease."[3]:840
Contents
Associated conditions
The disorder may coexist (in one quarter of cases) with polymyalgia rheumatica (PMR), which is characterized by sudden onset of pain and stiffness in muscles (pelvis, shoulder) of the body and is seen in the elderly. GCA and PMR are so closely linked that they are often considered to be different manifestations of the same disease process. Other diseases related with temporal arteritis are systemic lupus erythematosus, rheumatoid arthritis and severe infections.
Symptoms
It is more common in females than males by a ratio of 3:1. The mean age of onset is about 70 years, and it is rare in those less than 50 years of age.
Patients present with bruits:
- fever
- headache[4]
- tenderness and sensitivity on the scalp
- jaw claudication (pain in jaw when chewing)
- tongue claudication (pain in tongue when chewing) and necrosis[5]
- reduced visual acuity (blurred vision)
- acute visual loss (sudden blindness)
- diplopia (double vision)
- acute tinnitus (ringing in the ears)
The inflammation may affect blood supply to the eye and blurred vision or sudden blindness may occur. In 76% of cases involving the eye, the ophthalmic artery is involved causing anterior ischemic optic neuropathy.[6] Loss of vision in both eyes may occur very abruptly and this disease is therefore a medical emergency.
Diagnosis
Physical exam
- Palpation of the head reveals prominent temporal arteries with or without pulsation.
- The temporal area may be tender.
- Decreased pulses may be found throughout the body.
- Evidence of ischemia may be noted on fundal exam.
Laboratory tests
- Erythrocyte sedimentation rate, an inflammatory marker, >60 mm/hour (normal 10–40 mm/hour), but may be normal in approximately 20% of cases.
- C-reactive protein, another inflammatory marker, is also commonly elevated.
- Platelets may also be elevated.
Biopsy
The gold standard for diagnosing temporal arteritis is biopsy, which involves removing a small part of the vessel and examining it microscopically for giant cells infiltrating the tissue. Since the blood vessels are involved in a patchy pattern, there may be unaffected areas on the vessel and the biopsy might have been taken from these parts. Unilateral biopsy of a 1.5–3 cm length is 85-90% sensitive. So, a negative result does not definitely rule out the diagnosis.
Imaging studies
Radiological examination of the temporal artery with ultrasound yields a halo sign. Contrast enhanced brain MRI and CT is generally negative in this disorder. Recent studies have shown that 3T MRI using super high resolution imaging and contrast injection can non-invasively diagnose this disorder with high specificity and sensitivity.[7]
Treatment
Corticosteroids, typically high-dose prednisone (40–60 mg bd), must be started as soon as the diagnosis is suspected (even before the diagnosis is confirmed by biopsy) to prevent irreversible blindness secondary to ophthalmic artery occlusion. Steroids do not prevent the diagnosis from later being confirmed by biopsy, although certain changes in the histology may be observed towards the end of the first week of treatment and are more difficult to identify after a couple of months.[8] The dose of prednisone is lowered after 2–4 weeks, and slowly tapered over 9–12 months. Oral steroids are at least as effective as intravenous steroids,[9] except in the treatment of acute visual loss where intravenous steroids appear to offer significant benefit over oral steroids [10]
References
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- Giant Cell Arteritis article at University of Iowa
- Polymyalgia rheumatica article from National Institute of Arthritis and Musculoskeletal and Skin Diseases
es:Arteritis de células gigantes fr:Maladie de Horton it:Arterite temporale lv:Milzšūnu arterīts nl:Arteriitis temporalis no:Temporalisarteritt ja:側頭動脈炎 pl:Olbrzymiokomórkowe zapalenie tętnic pt:Arterite temporal sv:Hortons arterit
tr:Dev hücreli arterit- ↑ giant cell arteritis at Dorland's Medical Dictionary
- ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
- ↑ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
- ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
- ↑ Hayreh (April 3, 2003). "Ocular Manifestations of GCA". University of Iowa Health Care. Retrieved 2007-10-15.
- ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
- ↑ Font RL, Prabhakaran VC (2007). "Histological parameters helpful in recognising steroid-treated temporal arteritis: an analysis of 35 cases". The British journal of ophthalmology. 91 (2): 204–9. doi:10.1136/bjo.2006.101725. PMID 16987903.
- ↑ "BestBets: Steroids and Temporal Arteritis".
- ↑ Chan CC, Paine M, O'Day J.Br J Ophthalmol. 2001 Sep;85(9):1061-4.
